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Clinical manifestations and diagnosis of Paget disease of bone

Julia F Charles, MD, PhD
Section Editors
Ethel Siris, MD
Marc K Drezner, MD
Deputy Editor
Paul L Romain, MD


Paget disease of bone (PDB), also known historically as osteitis deformans, is a focal disorder of bone metabolism that occurs in the aging skeleton; it is characterized by an accelerated rate of bone remodeling, resulting in overgrowth of bone at single (monostotic PDB) or multiple (polyostotic PDB) sites and impaired integrity of affected bone. Commonly affected areas include the skull, spine, pelvis, and long bones of the lower extremity.

The majority of patients with PDB are asymptomatic. The diagnosis in such patients is usually made incidentally following a routine chemistry screen showing an elevated serum concentration of alkaline phosphatase of bone origin or an imaging study obtained for some other reason that shows pagetic changes in bone.

The two main clinical manifestations of Paget disease are pain due to the pagetic lesion in bone itself or from secondary consequences of bone overgrowth and deformities in affected areas, such as osteoarthritis or nerve impingement. Fractures, bone tumors, neurologic disease, and abnormalities in calcium and phosphate balance can also occur. In addition, because of the vascularity of pagetic bone, excessive bleeding may occur during orthopaedic surgery.

The pathogenesis, clinical manifestations, and diagnosis of PDB will be reviewed here. The treatment of this disorder is discussed elsewhere. (See "Treatment of Paget disease of bone".)


Paget disease of bone (PDB) is a fairly common finding in aging bone, with estimates ranging from 2.3 to 9 percent in older patients within affected populations [1,2]; it is often asymptomatic. Its onset is typically after age 55, with a slight predominance in men in some but not all studies. It is common in England, Scotland, Central Europe, and Greece, as well as in countries and cities settled by European immigrants, such as those in Australia, Canada, and the United States. Even within these countries, there are geographical clusters of disease [1,3-7]. Paget disease is rarely reported in the Scandinavian countries and Asia [1,8-11].

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Literature review current through: Nov 2017. | This topic last updated: Jul 18, 2016.
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